Vulvar sarcomas: Short guideline for histopathological recognition and clinical management. Part 2

Our plain-English summary of Vulvar sarcomas: Short guideline for histopathological recognition and clinical management. Part 2, by A.A. Chokoeva, G. Tchernev, J.C. Cardoso, et al (International Journal of Immunopathology and Pharmacology, 2015).

The study

• Authors: A.A. Chokoeva, G. Tchernev, J.C. Cardoso, J.W. Patterson, I. Dechev, S. Valkanov, M. Zanardelli, T. Lotti, U. Wollina
• Journal: International Journal of Immunopathology and Pharmacology
• Published: 2015;28(2):178-86
• Type of article: Editorial, Review
• Cited by: 14 articles, as counted by NIH iCite
• Read it: PubMed · Publisher

Abstract

Malignant tumors of the female reproductive system are a serious health and social problem, as they are the second most common cause of death among women, after breast cancer. Vulvar tumors represent only 4% of all gynecological neoplasms, and they are fourth in frequency after tumors of the cervix, uterus, and ovary. Ninety-eight percent of all vulvar tumors are benign and only 2% are malignant. Sarcomas of the vulva comprise approximately 1-3% of all vulvar cancers. They are characterized by rapid growth, high metastatic potential, frequent recurrences, aggressive behavior, and high mortality rate. In Part 1 of this paper, we presented the most common forms of sarcoma of the vulva: leiomyosarcoma, epithelioid sarcoma, malignant rhabdoid tumor, and rhabdomyosarcoma. The second part of this review will focus mainly on the rarest variants of vulvar sarcoma: low-grade fibromyxoid sarcoma, synovial sarcoma, monophasic synovial sarcoma, carcinosarcoma, Ewing sarcoma, myeloid sarcoma, dermatofibrosarcoma protuberans, malignant fibrous histiocytoma, angiomatoid fibrous histiocytoma, liposarcoma, malignant peripheral nerve sheath tumor, and malignant mesothelioma.

© The Author(s) 2015.

In plain English

This article was the second part of a two-part review of sarcomas (cancers of connective tissue) of the vulva. It did not study new patients. Instead it summarized published reports on rare vulvar sarcomas, including synovial sarcoma, carcinosarcoma, Ewing sarcoma, myeloid sarcoma, dermatofibrosarcoma protuberans, malignant fibrous histiocytoma, liposarcoma, malignant peripheral nerve sheath tumor and malignant mesothelioma. Asbestos appeared only in the short mesothelioma section. Citing earlier work, that section said malignant mesothelioma usually occurs in the pleura, less often in the peritoneum and tunica vaginalis testis, and that asbestos exposure is considered a predisposing factor. No fiber type was named. The review described vulvar mesothelioma as extremely rare, known from anecdotal cases in the labia minora that presented as a 3–4 cm encapsulated soft tumor. It said no specific immunohistochemical panel confirms the diagnosis, and that treatment consisted of wide excision with radiotherapy after surgery. The review did not say whether the vulvar cases had asbestos exposure. The authors concluded that vulvar sarcomas are rare, often misdiagnosed and aggressive, and should be considered when diagnosing non-specific vulvar lesions.

We wrote this plain-English summary from the full article with the help of AI, and a second AI model checked each sentence against the article. It is not the authors' wording; the abstract and the article are their own account.

What the study did and found

• Design: Narrative literature review (Part 2 of two parts); no original patient data
• Who was studied: No study population; the authors reviewed published literature, largely case reports, on rare sarcomas of the vulva
• Exposure: Not the subject of the review. Asbestos is mentioned once, citing other sources, as a predisposing factor for malignant mesothelioma in general; no fiber type is named
• Main results:
  • Citing other sources, malignant mesothelioma was described as usually occurring in the pleura, less frequently in the peritoneum and tunica vaginalis testis
  • Malignant mesothelioma of the vulva was described as extremely rare, reported as anecdotal cases presenting in the labia minora
  • In these reported cases the tumor presented as a 3–4 cm encapsulated soft tumor in the superior and outer margin of the labium minus
  • No specific immunohistochemical panel confirms the diagnosis. The tumors reacted with EMA, mesothelin-1 and HBME-1 and were negative for CEA and CD15
  • Treatment of vulvar mesothelioma was described as wide excision with postoperative radiotherapy to prevent recurrences, which were described as rare
• The authors' conclusion: The authors concluded that vulvar sarcomas are rare, often misdiagnosed, aggressive tumors with poor prognosis, and that they should be considered in the differential diagnosis of non-specific vulvar lesions

Topics

Medical terms in this study, explained in our Lexicon: Sarcoma, Neoplasm, Epithelioid, Rhabdoid, Synovial Sarcoma, Malignant Mesothelioma.

Related studies

• Soft tissue sarcomas of vulva — I. Davos, M.R. Abell (1976)
• Clinical results in the management of patients with uterine sarcoma — K.J. Lohe, J. Baltzer, et al. (1980)
• Benign multicystic mesothelioma and peritoneal inclusion cysts: are they the same clinical and histopathological entities? A systematic review to find an evidence-based management — A.M.C. Rapisarda, A. Cianci, et al. (2018)
• Epithelioid sarcoma. A clinical and pathologic study of nine cases — H. Santiago, L.K. Feinerman, et al. (1972)
• Diagnostic surgical pathology in lung cancer: Diagnosis and management of lung cancer, 3rd ed: American College of Chest Physicians evidence-based clinical practice guidelines — A.M. Schwartz, M.K. Rezaei (2013)
• Sarcomas in organ allograft recipients — I. Penn (1995)

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